Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases

Grothues J, Hardes J, Agaimy A, Collaud S, Podleska LE, Farzalyev F, Engel NM, Hamacher R, Fletcher B, Pöttgen C, Bertram S, Schildhaus HU, Streitbürger A, Bauer S, Falkenhorst J (2024)

Publication Language: English

Publication Type: Journal article

Publication year: 2024

Journal

Journal of Cancer Research and Clinical Oncology Springer Verlag (Germany)

Book Volume: 150

Article Number: 494

Journal Issue: 11

DOI: 10.1007/s00432-024-05980-3

Abstract

Purpose: Clear cell sarcoma (CCS) of tendons and aponeuroses and CCS-like malignant gastrointestinal neuroectodermal tumor/sarcoma (GINET) are characterized by frequent local and distant relapses, alongside with low efficacy of all systemic treatments. We aimed to collect a comprehensive dataset to identify prognostic factors and treatment outcomes. Methods: We performed a retrospective single center analysis for diagnosed CCS and GINET on demographic, tumor, treatment and survival data. Results: We identified 43 patients (w:25, m:18) with a median follow-up of 35mo and a 5y-OS-rate of 42%. At diagnosis the median age was 42yrs. Median tumor size was 3.6 cm (0.3–11.1 cm), and 24/26 (94%) tissues analyzed at our institute were EWSR1::ATF1-translocation-positive. Distant extremities (incl. knee or elbow) were affected in 72.5%. Of note, 79.5% received an excisional biopsy (benign histology suspected in 30.2%) leading to frequent incomplete resection. Final R0 status correlated significantly (p = 0.017) with longer survival rates compared to R + status in localized CCS (N0M0, 5-yr OS 0% vs 64%). Radiation and systemic treatment had limited antitumor effects while isolated limb perfusion was active in some patients. 18.6% of patients showed lymphatic spread and 20.9% distant metastases. Presence of initial M + was associated with a dismal survival of 1.4 years (M +) vs 7.1 years (M0; p <.001). Conclusion: We here present one of the largest clinical cohorts of patients with CCS/GINET. Our data underscores the exceptional risk of metastatic disease even in small tumors. As systemic treatment and radiation showed limited efficacy, complete resection was the most important treatment option.

Authors with CRIS profile

Abbas Agaimy Lehrstuhl für Allgemeine Pathologie und Pathologische Anatomie

Involved external institutions

Universität Witten/Herdecke DE Germany (DE) Universitätsklinikum Essen DE Germany (DE) Institut für Pathologie Nordhessen DE Germany (DE)

How to cite

APA:

Grothues, J., Hardes, J., Agaimy, A., Collaud, S., Podleska, L.E., Farzalyev, F.,... Falkenhorst, J. (2024). Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases. Journal of Cancer Research and Clinical Oncology, 150(11). https://doi.org/10.1007/s00432-024-05980-3

MLA:

Grothues, Janik, et al. "Prognostic factors in clear cell sarcoma: an analysis of soft tissue sarcoma in 43 cases." Journal of Cancer Research and Clinical Oncology 150.11 (2024).

BibTeX: Download